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Rhabdomyosarcomas (RMS) form a major subset of pediatric sarcomas with an incidence of 4.5/million cases/year. Adult RMS is rare. Retroperitoneal (RP) RMS in adults is rarer with only a handful of cases reported to date. The management involves surgery with chemotherapy for resectable disease and definitive chemoradiotherapy for the unresectable. The outcome of adult RP RMS is worse than the pediatric counterparts. This image illustrates a locally advanced adult RP RMS treated with perioperative chemotherapy and multivisceral along with vascular resection to achieve a favorable outcome.
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